Background Vogt-Koyanagi-Harada (VKH) syndrome is a multisystem disorder characterized by granulomatous panuveitis with exudative retinal detachments that is often associated with neurologic and cutaneous manifestations. Aim of the work The aim of this case report is to describe a rare case with Vogt-Koyanagi-Harada syndrome that developed an explosive form of reactive arthritis shortly after attack of Chlamydial urethritis. An association between Vogt-Koyanagi-Harada and ulcerative colitis was previously described in several case reports. The case is described in detail and the literature was reviewed. Case report In this report we described a male patient with long standing Vogt-Koyanagi-Harada syndrome, who developed aggressive reactive arthritis two weeks after an attack of Chlamydial urethritis. Clinically the patient presented with bilateral sacroiliitis, peripheral arthritis, and wide spread enthesitis. The patient had positive family history of scleroderma in his first degree relative and HLA-B27 testing was negative. Conclusion In this report we theoretically proposed a possible relationship between VKH and Seronegative spondyloarthropathy group of disorders.